[Pulmonary infections in cystic fibrosis: pathogenesis and therapy]

Abstract

Infections of airways and lung in patients with cystic fibrosis determine quality of life and prognosis. Despite overall improvement of management of infections the underlying causes leading to infection early in life remain an enigma. As a consequence of infection various morphologic alterations arise. The most prominent are development of bronchiectases afflicting more than 70% of patients at age two. The spectrum of bacterial involvement has undergone significant changes. In contrast to earlier reports Pseudomonas aeruginosa at present is the most commonly encountered pathogen. Mucoid forms are typical for cystic fibrosis and are rarely seen in other conditions. Pseudomonas cannot be eradicated once it is established. Antipseudomonas chemotherapy leads to a diminution of bacteria from 10(8)/ml sputum to 10(6) at best. However, clinical results are convincing. Thus regular antispeudomonas treatment has been advocated by one CF-centre. Apart from conventional chemotherapy alternative approaches of treatment such as vaccination or immunoregulation need to be explored in greater detail.